Orbital granulocytic sarcoma
a Department of
Ophthalmology, McGill University, Montreal, Canada, b Instituto de
Oftalmobiologia, Universidad de Valladolid, Valladolid, Spain, c Cullen Eye Institute, Baylor College of Medicine,
Houston, USA
Correspondence to: Dr M N Burnier, Department of Ophthalmology, Royal Victoria Hospital, 687 Pine Avenue West, Room H7, Montreal, Quebec, Canada, H3E 1A1.
Accepted for publication 13 August 1997
AIM
Orbital granulocytic sarcoma is a localised
tumour composed of cells of myeloid origin. Histological diagnosis can
be difficult in patients with poorly differentiated orbital tumours and
no evidence of systemic leukaemia. The naphthol AS-D chloracetate esterase (Leder stain) and immunohistochemical stains for lysozyme and MAC387 were used to determine the staining characteristics of these
tumours. A case series of seven patients with orbital granulocytic
sarcoma is presented.
METHODSSeven patients with orbital granulocytic
sarcoma were studied. Haematoxylin and eosin, Leder, and lysozyme
stained sections were available in seven cases. Unstained formalin
fixed paraffin embedded sections of seven cases were available for
immunohistochemical evaluation using the avidin-biotin-complex
technique for MAC387.
RESULTSThe mean age of presentation of the
orbital tumour was 8.8 years. Four patients presented with an orbital
tumour before any systemic manifestations of leukaemia. In two cases
the diagnosis of the orbital tumour and systemic leukaemia was made
simultaneously. There was one case of established systemic myeloid
leukaemia in remission with the subsequent development of orbital
granulocytic sarcoma. Six of seven cases (86%) were positive for the
Leder stain. Five of seven cases (71%) showed positive
immunoreactivity with lysozyme. The immunohistochemical stain for
MAC387 was positive in all seven cases (100%) including one case that
was negative for both lysozyme and Leder stains.
CONCLUSIONSOrbital granulocytic sarcoma is a
tumour that affects children and can present with rapidly progressive
proptosis. This tumour may develop before, during, or after the
occurrence of systemic leukaemia. The combination of Leder and lysozyme
stains is useful in the diagnosis of orbital granulocytic sarcoma.
MAC387 may be a more reliable marker for orbital granulocytic sarcoma.
© 1997 by British Journal of Ophthalmology
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